Journal
RHEUMATOLOGY
Volume 57, Issue 4, Pages 712-717Publisher
OXFORD UNIV PRESS
DOI: 10.1093/rheumatology/kex458
Keywords
systemic sclerosis; anti-nuclear antibody; anti-eIF2B; anti-RuvBL1/2; ANA-negative; interstitial lung disease; prognosis
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Funding
- Scleroderma Raynaud's UK
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Objectives. Autoantibodies targeting ubiquitously expressed nuclear antigens can be identified in most patients with SSc. Cytoplasmic autoantibodies (in otherwise ANA-negative sera) targeting eukaryotic initiation factor-2B (anti-eIF2B) have recently been identified in SSc with clinical associations to dcSSc disease and interstitial lung disease (ILD), although the majority of samples originated from a tertiary SSc-ILD centre. We investigated the prevalence and clinical associations of recently described SSc-specific (including anti-eIF2B) and other cytoplasmic autoantibodies in ANA-negative sera obtained from a large representative SSc cohort. Methods. ANA-negative sera from the Scleroderma Family Registry and DNA Repository underwent indirect immunofluorescence, radiolabelled protein immunoprecipitation (+/- immunodepletion) to identify anti-eIF2B and other CTD-related autoantibodies. The clinical phenotype of positive samples was evaluated. Results. Immunoprecipitation was performed on 128 ANA-negative samples (obtained from 3249 SSc patients). Anti-eIF2B antibodies were present in nine patients (7%), the majority of whom had dcSSc (8/9). SSc-ILD was present in all anti-eIF2B patients for whom chest imaging was available (7/9). Anti-synthetase autoantibodies (targeting PL12, PL7, OJ and Zo) were identified in seven patients (5.5%), all of whom fulfilled the 2013 ACR/EULAR classification criteria for SSc and had evidence of SSc-ILD where relevant outcomes were available for evaluation. Anti-RuvBL1/2 antibodies were identified in two patients with SSc-overlap syndromes. Conclusion. Anti-eIF2B antibodies are cytoplasmic SSc-specific autoantibodies with strong clinical associations with dcSSc and SSc-ILD found in ANA-negative sera. Anti-synthetase autoantibodies, and other recently discovered SSc-specific antibodies such as anti-RuvBL1/2, can also be identified in ANA-negative SSc.
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